Hospital Affiliation

Zollinger-Ellison Syndrome

Definition

Zollinger-Ellison syndrome is a rare disorder that arises from tumors and causes ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). Not only can these tumors lead to ulcers, they can also be cancerous, and spread to the nearby lymph nodes or liver.

About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). People with MEN 1 may have additional endocrine tumors in the brain and neck.

Causes

Zollinger-Ellison syndrome is caused by gastrin-producing tumors, called gastrinomas. Gastrin is a hormone that stimulates the stomach to produce acid. Excess acid production may lead to ulcers
in the
stomach
or
duodenum.

Risk Factors

Factors that may increase your chance of Zollinger-Ellison syndrome include:

Revision Information

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

Berna MJ, Hoffmann KM, et al. Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National institutes of Health and comparison with 537 cases from the literature, evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features.
Medicine. 2006;85:331-364.